catalog number :
MBS512134
products full name :
Goat anti-human Protein S (PS), Peroxidase Conjugated IgG
products short name :
Protein S
products name syn :
Protein S, human
other names :
Protein S; Protein S; vitamin K-dependent protein S; protein Sa; OTTHUMP00000197145; OTTHUMP00000197146; vitamin K-dependent plasma protein S; protein S (alpha)
other gene names :
PROS1; PROS1; PSA; PROS; PS21; PS22; PS23; PS24; PS25
uniprot entry name :
Q8IXC8_HUMAN
specificity :
Prior to conjugation, this antibody was specific for Protein S as demonstrated by immunoelectrophoresis and ELISA.
purity :
Peroxidase conjugated IgG. Vial containing ml of whole IgG conjugated to horseradish peroxidase (HRP) through carbohydrate groups. Total protein is 0.2 mg.
form :
IgG-HRP conjugate as a clear, slightly red-brown liquid.
concentration :
IgG-HRP concentration is mg/ml, determined by absorbance using an extinction coefficient (E1%280) of 14.
storage stability :
Store between -10 and -20 degree C. Product will become viscous but will not freeze. Avoid storage in frost-free freezers. Keep vial tightly capped. Allow product to warm to room temperature and gently mix before use. Avoid exposure to sodium azide as this is an inhibitor of peroxidase activity.
app notes :
Suitable as a source of peroxidase labelled antibodies to PS.
other info1 :
Immunogen: Human Protein S purified from plasma. Rz Ratio (Reinheitszahl. A403/A280): 0.39 as determined spectrophotometrically.
other info2 :
Buffer: A buffered stabilizer solution containing 50% (v/v) glycerol.
products description :
Protein S (PS) is a vitamin K-dependent glycoprotein produced in the liver, endothelium and megakaryocytes. The concentration of PS in plasma is ~25 ug/ml (~325 nM) where it acts as a cofactor in the anticoagulant activity of activated Protein C. A deficiency of Protein S (quantitative or qualitative) is a risk factor for vascular thrombosis. Protein S is expressed as a single chain molecule with a molecular weight of 77 kDa. The structure of PS is similar to many other vitamin-K dependent coagulation proteins, consisting of an Nterminal calcium binding domain of 10 gamma-carboxyglutamic acid (gla) residues, followed by a thrombin-sensitive loop region and 4 EGF-like domains. The C-terminal domain does not contain the usual catalytic triad of a proenzyme, but seems instead to be involved in the binding of PS to C4b-binding protein (C4bp). Protein S binds to activated Protein C (APC) in the presence of calcium and negatively charged phospholipid surface to allow APC to proteolytically inactivate coagulation cofactors Va and VIIIa. Enzymatic regulation of PS cofactor activity is through cleavage of PS in the thrombin-sensitive loop region by thrombin or other enzymes, resulting in the loss of calcium binding properties and APC cofactor activity. Another regulatory mechanism is to reduce the availability of PS by the binding of PS to C4bp. In plasma, approximately 60% of Protein S circulates in non-covalent complex with C4bp, making it unavailable for APC cofactor activity. The binding of PS to C4bp may be important in localizing C4bp to damaged cell membranes where it may control activation of complement by the classical pathway1-3.
products references :
1. Broze GJ, Miletich JP; Biochemistry and Physiology of Protein C, Protein S and Thrombomodulin; in Hemostasis and Thrombosis, 3rd Edition, eds. RW Colman, J Hirsh, VJ Marder and EW Salzman, pp 259-276, J.B. Lippincott Co., Philadelphia PA, USA, 1994. 2. Comp PC, Doray D, Patton D, Esmon CT; An Abnormal Plasma Distribution of Protein S Occurs in Functional Protein S Deficiency. Blood 67, pp 504- 508, 1986. 3. Schwalbe RA, Dahlback B, Nelsestuen GL; Independent Association of Serum Amyloid P Component, Protein S and Complement C4b with C4bbinding Protein and Subsequent Association of the Complex with Membranes; JBC 265, pp 21749-21757, 1990.
ncbi acc num :
BAC54142.1
ncbi gb acc num :
AB083394.1
ncbi mol weight :
4,488 Da
ncbi pathways :
Cell Surface Interactions At The Vascular Wall Pathway (106062); Common Pathway (106060); Complement And Coagulation Cascades Pathway (198880); Complement And Coagulation Cascades Pathway (83073); Complement And Coagulation Cascades Pathway (484); Formation Of Fibrin Clot (Clotting Cascade) Pathway (106057); Formation Of Platelet Plug Pathway (106029); Gamma-carboxylation Of Protein Precursors Pathway (106233); Gamma-carboxylation, Transport, And Amino-terminal Cleavage Of Proteins Pathway (106232); Hemostasis Pathway (106028)
ncbi summary :
This gene encodes a vitamin K-dependent plasma protein that functions as a cofactor for the anticoagulant protease, activated protein C (APC) to inhibit blood coagulation. It is found in plasma in both a free, functionally active form and also in an inactive form complexed with C4b-binding protein. Mutations in this gene result in autosomal dominant hereditary thrombophilia. An inactive pseudogene of this locus is located at an adjacent region on chromosome 3. [provided by RefSeq]
uniprot summary :
PROS1: Anticoagulant plasma protein; it is a cofactor to activated protein C in the degradation of coagulation factors Va and VIIIa. It helps to prevent coagulation and stimulating fibrinolysis. Defects in PROS1 are the cause of thrombophilia due to protein S deficiency, autosomal dominant (THPH5). A hemostatic disorder characterized by impaired regulation of blood coagulation and a tendency to recurrent venous thrombosis. However, many adults with heterozygous disease may be asymptomatic. Based on the plasma levels of total and free PROS1 antigen as well as the serine protease-activated protein C cofactor activity, three types of PROS1D have been described: type I, characterized by reduced total and free PROS1 antigen levels together with reduced anticoagulant activity; type III, in which only free PROS1 antigen and PROS1 activity levels are reduced; and the rare type II which is characterized by normal concentrations of both total and free PROS1 antigen, but low cofactor activity. Defects in PROS1 are the cause of thrombophilia due to protein S deficiency, autosomal recessive (THPH6). A very rare and severe hematologic disorder resulting in thrombosis and secondary hemorrhage usually beginning in early infancy. Some affected individuals develop neonatal purpura fulminans, multifocal thrombosis, or intracranial hemorrhage. Protein type: Secreted; Secreted, signal peptide. Chromosomal Location of Human Ortholog: 3q11.2. Cellular Component: Golgi membrane; extracellular space; endoplasmic reticulum membrane; Golgi lumen; extracellular region; plasma membrane. Molecular Function: endopeptidase inhibitor activity; calcium ion binding. Biological Process: platelet activation; fibrinolysis; cellular protein metabolic process; platelet degranulation; regulation of complement activation; innate immune response; proteolysis; post-translational protein modification; blood coagulation; leukocyte migration; peptidyl-glutamic acid carboxylation. Disease: Thrombophilia Due To Protein S Deficiency, Autosomal Recessive; Thrombophilia Due To Protein S Deficiency, Autosomal Dominant